Your Digest for Thursday, Mar 14, 2024 11:59 PM


[!INFO] Definitions:
left ventricular hypertrophy = increased left ventricular mass.
Hypertrophy is divided into concentric and eccentric based on the "relative ventricular wall thickness".
This in turn is defined as "ventricular wall thickness divided by LV diastolic diameter".

Congenital infections

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Source
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Vertical transmission can occur from other to baby by

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A) Infections acquired transplacentally:

[!INFO] Mnemonic CLAP TORCHES

  1. CLAP - Chickekpox, Listerial, AIDS, Parvovirus b19
  2. ToRCHES - Toxoplasmosis, Rubella, CMV, Herpes, Enterovirus, Syphilis
  3. Also Zika virus

B) Infections acquired from birth canal

[!INFO] Mnemonic: 3 viruses, 5 bacteria

[!INFO] Viruses which only rarely cross the placenta
HIV, HSV, Hep B, EBV.
More frequently, they are transmitted perinetally

Decription of transplacental infections

#TODO read routes of infection from this source.

Toxoplasmosis Rubella CMV Herpes Enterovirus Syphillis
CCCc BCDG All S's
Chorioretinitis
Convulsions
Calfications
cephalus - hydro / micro
Blindnes
Cataracts
Deafness
Growth impairment
T1: Microcephaly, seizures, chrorioretinitis
Later: Hepatitis, pneumonia
Mucocutaneous vessicles
Conjunctivitis
Other organs (seizurse, ⬆liver enzymes)
myocarditis
Encephalitis
Hearing loss
Skin
Skeletal
Splenomegaly
Saddle nose
Smile (Hutchison's teeth)
Highest risk - T1 (50-90% risk) Commonest congenital infection (30% risk)
Chlamydia Listeria AIDS Parvovirus
Conjunctivitis
Pneumonia
Amnionitis
Neonatal sepsis
Neonatal death or hydrops; but most do not have adverse outcomes. (UpToDate)

Congenital varicella infection

Presentation depends on time of infection:

  1. mother is infected before POA of 20 -> neonatal varicella syndrome
    1. Scars - cicatrix, Limb hypoplasia, cataracts, chorioretinitis , microcephalus, seizures
    2. prevent with VZIG (give empirically if mother's varicella antibody status cannot be determined within 96 hours) Source
  2. Mother develops vessicles -5 to +2 days after delivery:
    1. severeNeonatalVaricella.png
    2. (because the foetus doesn't have time to acquire protective maternal antibodies)
    3. Severe foetal varicella infection
    4. Give VZIG to mother and VZIG to baby upon delivery (or as soon as mother develops vesicles)

[!INFO] mnemonic: patient numbers
Phase I - double digits
Phase II - Triple digits
Phase III - quadruple digits, also C being the 3rd letter of the alphabet, phase III trials 'compare' efficacy -> RCTs.


[!INFO] The Big 5 STIs
Chlamydia, gonorrhoea, Syphilis, HIV, hepatitis B,
and trichomoniasis.


Rabies post exposure prophylaxis

[[AntirabiesPostExposure.pdf]]
2 site intradermal - 2,2,2,0,2
on day 0,3,7,30

4 site intradermal is similar - 4,2,2,0,2
recommended for late presentaion, border line exposure or major exposure from observable healthy domestic animal.

IM arv course is on day 0,3,7,14,30.
something called the 2-2-1 regimen is used for minor exposurse.


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Macrophage activation syndrome (MAS)⭐


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Caused by HHV-4. (human herpevirus 4) [[viralClassificationFamilies.png]]


MCD in adults:
- NSAID induced
- NSAIDS can cause haemodynamically mediated AKI and Acute Interstitial nephritis - which can lead to MCD.Source
- Lithium, mercury - (both also a cause for membranous nephropathy)
- Malignancies, particulartly haematologic ones: Hodgkin lymphoma and non hodgkin lymphoma (paraneoplastic)

FSGS (Focal segmental glomerulosclerosis)

Primary FSGS Secondary FSGS
Sometimes responds to steroids, failure of steroids is common. Other immunosuppresants are used. Usually poor response to steroids. ACEi are better
Presents as massive proteinuria, haematuria and hypertension. Can be caused by any process which reduces functioning number of nephrons (eg. nephrectomy)
(nephrectomy, hypertension, gross obesity, IgA nephropathy, HIV, CMV, EBV, hepatitis B and C)
Lithium, Heroin, bisphosphonates (Pamidronate), anabolic steroids,
reflux nephropathy
Sickle cell disease [[Haematology MCQ discussion#Q5 Sickle cell disease]]
Source
- ![SelectiveVsNonSelectiveProteinLossInGlomerulonephritis.png](SelectiveVsNonSelectiveProteinLossInGlomerulonephritis.png)
- Electrophoresis of urine samples in different diseases: [Source](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6874928/)
- Despite this electrophoresis clearly showing that MCD doesn't loose much IgG, *infection is a well known complication of minimal change disease*. [Source](https://emedicine.medscape.com/article/244631-overview#showall)(maybe due to complement loss, steroids etc)

Membranous glomerulonephritis

Membranous nephropathy -
- Need to exclude hepatitis B, SLE, thyroiditis, some drugs (penicillamine, gold / mercury, captopril) and malignancy.
- Associated malignancies: prostate, lung, lymphoma, leukemia
Prognosis - rule of thirds (passMedicine)


⭐Charcot Marie Tooth disease

[!TIP] Mnemonic:
Marie : Odd ones out (odd numbers affect the 'external' myelin


[!INFO] see also
[[General Medicine 4#Q 12 Rabies prophylaxis|Rabies prophylaxis]]

Eosinophillic, **cytoplasmic** inclusions of viral proteins visible in the *hippocampus and cerebellum*. 

PKD genes encode the polycystin proteins. Mutations of these cause decreased intracellular calcium in the principal cells of the collecting duct -> dysoriented cell division -> cyst formation.
Polycystin 1 (in PKD1) is a regulator of tubular epithelial cell adhesion and differentiation. Polycystin 2 (in PKD2) is an ion channel. These are required for function of cilia of the tubular epithelium which are in turn required for flow sensing. Impaired flow sensing causes a defect in proliferation and differentiation -> cysts.
Cyst formation occurs only in 5-10% of nephrons but that's enough to cause renomegaly.

Caplan Syndrome


Hookworm

[!TIP] Mnemonic:
Hookworm - larva migrans.(slow)
Strongyloides -> larva currens (fast)
Strongyloides are strong -> therefore move fast -> "currens"
larvaCurrensVsLavaMigrans.jpg

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Proteins which are normally soluble start getting deposited extracellularly as beta pleted fibrils.
1. Dialysis-related amyloidosis (DRA) is a disabling disease characterized by accumulation and tissue deposition of amyloid fibrils consisting of beta2-microglobulin (beta2-m) (Aβ2M) in the bone, periarticular structures, and viscera of patients with end-stage kidney disease. Beta2-m is a component of the major histocompatibility complex that is present on cell surfaces and is normally cleared by glomerular filtration
2. Aβ2M amyloidosis presents with carpal tunnel syndrome.
amyloidosisEvaluationAlgorithm.png

[!INFO] Once the presense of amyloid is found
amyloidPrecursorProtein.png
the precursor protein must be determined.
(Both (AL and AA) have the beta pleted sheet structure) (Harrison's - page 804)
| Type | Constituent |
| ------------------------- | --------------------------------------------------------------------------------------------------------------------------------- |
| AL Amyloidosis | Deposition of Ig Light chain fragments |
| Transthyretin amyloidosis | |
Commonest involved organs:

  1. Kidney
  2. heart

The symptoms and signs of AL and AA cannot reliably be distinguished. - Harrison's

AA amyloido is derived from a serum precursor called serum amyloid associated protein. (SAA protein).
It is made in the liver under the stimulation of IL-1, and IL-6.

A-beta amyloid (Aβ amyloid)

Found in Alzheimer's disease.
The precursor for this is Amyloid precursor proteins. (APP)


- *Probenecid* - avoid in renal impairment
- **Anakinra** - *IL-1 inhibitor*; very expensive.

[!WARNING]
Hypoparathyroidism in the image is probably incorrect; most sources say hyperparathyroidism.

P is for positive - peudogout pyrophosphate crystals show positive birefrigence.

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Gout Pseudogout
Uric acid crystal Calcium pyrophosphate
Needle shaped, negatively birefingent Rhomboid shaped, positively birefringent
Associated with triggers (heavy meal, alcohol, illness, dehydration) No acute triggers
Risk factors: Joint damange, OA, increased age
also associated with GOUT, hyperparathyroidism, hypomagnesemia, haemochromatosis
Joint fluid can look purulent.
Commoner in males Male = female.
30 - 60 years Older - ? 70 years
Uric acid levels fall immediately after an episode
Commonest joint: MTP, also wrist, ankle knee, elbow, wrist, ankle
#2015MCQ/Q35
- (?not following viral infection)
- Also plantar fasciits, achilles tendinits, balanitis, anterior uveitis

^36d6c8

[!TIP] Mnemonic: TAMPAX -> "TAMPOX"

TAMponade - Pulsus paradoxus and only X descent are seen. (Y descent is absent)
Constrictive -> "Konsrictive" -> Kussmaul sign [[#^36d6c8]] is present in constrictive pericarditis.
| Left and Right ventricular diastolic pressure and left and right atrial pressures (PCWP) all rise to match the pericardial pressure. (i.e ?no pressure gradient from atrium to ventricle -> impaired flow across TV -> absent Y descent) | ?Greatly increased ventricular interdependance |



Always over 50;


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[!INFO] The only enterovirus we have a vaccine for is polio virus.